Biliary atresia is characterized by obliteration or discontinuity of the extra hepatic biliary system, resulting in obstruction to bile flow. There are 3 types of biliary atresia:
Type 1: Atresia restricted to common bile duct
Type 2: Atresia of the common hepatic duct
Type 3: Atresia of the right and left hepatic duct
Patients with biliary atresia generally pass acholic stools with onset at about 2 weeks of life. They are average birth weight. They have firm hepatomegaly. {1} There is a female predominance. They may have associated polysplenia syndrome, heterotaxy, and reverse rotation of intestine, in isolation or in various combinations and intra-abdominal vascular anomalies. {2} In addition, malrotation, Meckel’s diverticulum and jejunal atresia have also been reported with biliary atresia. {3} Biliary atreisa in association with other congenital structural anomalies may have a poor prognosis. These patients have poor bile secretion after hepatic portoenterostomy. {4}
References
1. Shah I, Parikh S. Clinical and Biochemical Factors Associated With Biliary Atresia. Tropl Gastroenterol. 2012` 33: 214-217
2. Rasool F, Mirza B. Polysplenia syndrome associated with situs inversus abdominus and type I jejunal atresia. APSP J Case Rep. 2011` 2: 18
3. Kataria R, Kataria A, Gupta DK. Spectrum of congenital anomalies associated with biliary atresia. Indian J Pediatr. 1996`63:651-654
4. Tanano H, Hasegawa T, Kawahara H, Sasaki T, Okada A. Biliary atresia associated with congenital structural anomalies. J Pediatr Surg. 1999` 34: 1687-90
| References : |
- Shah I, Parikh S. Clinical and Biochemical Factors Associated With Biliary Atresia. Tropl Gastroenterol. 2012; 33: 214-217
- Rasool F, Mirza B. Polysplenia syndrome associated with situs inversus abdominus and type I jejunal atresia. APSP J Case Rep. 2011; 2: 18
- Kataria R, Kataria A, Gupta DK. Spectrum of congenital anomalies associated with biliary atresia. Indian J Pediatr. 1996;63:651-654
- Tanano H, Hasegawa T, Kawahara H, Sasaki T, Okada A. Biliary atresia associated with congenital structural anomalies. J Pediatr Surg. 1999; 34: 1687-90
|